: Erdheim-Chester disease is an uncommon non-Langerhans cell histiocytosis affecting multiple systems. There is limited knowledge on the imaging capabilities of this disease. We present an extremely rare case of Erdheim-Chester illness in a 67-year-old man with multisystem involvement, including the cardiovascular system, skeleton, retroperitoneum (renal and adrenal infiltration) and the neurologic system. The involvement of the various organs was thoroughly assessed using multimodal imaging modalities such as computed tomography, magnetic resonance imaging, positron emission tomography and bone scintigraphy. Erdheim-Chester illness was revealed by a bone biopsy. Especially when there is cardiac and cerebral involvement, Erdheim-Chester illness is a rare condition with a poor prognosis. Knowing the imaging characteristics of Erdheim-Chester disease may be helpful in understanding the radiological results of many organs affected by the disease as described and discussed in the current case report.

Chiocchi, M., Luciano, A., De Stasio, V., Pugliese, L., Di Donna, C., Cerocchi, M., et al. (2023). Radiological findings in Erdheim Chester disease: A very rare multisistemic disease. RADIOLOGY CASE REPORTS, 18(5), 2047-2054 [10.1016/j.radcr.2023.02.063].

Radiological findings in Erdheim Chester disease: A very rare multisistemic disease

Chiocchi, Marcello;Garaci, Francesco;Floris, Roberto
2023-05-01

Abstract

: Erdheim-Chester disease is an uncommon non-Langerhans cell histiocytosis affecting multiple systems. There is limited knowledge on the imaging capabilities of this disease. We present an extremely rare case of Erdheim-Chester illness in a 67-year-old man with multisystem involvement, including the cardiovascular system, skeleton, retroperitoneum (renal and adrenal infiltration) and the neurologic system. The involvement of the various organs was thoroughly assessed using multimodal imaging modalities such as computed tomography, magnetic resonance imaging, positron emission tomography and bone scintigraphy. Erdheim-Chester illness was revealed by a bone biopsy. Especially when there is cardiac and cerebral involvement, Erdheim-Chester illness is a rare condition with a poor prognosis. Knowing the imaging characteristics of Erdheim-Chester disease may be helpful in understanding the radiological results of many organs affected by the disease as described and discussed in the current case report.
mag-2023
Pubblicato
Rilevanza internazionale
Articolo
Esperti anonimi
Settore MED/36 - DIAGNOSTICA PER IMMAGINI E RADIOTERAPIA
English
Cerebellar atrophy
Erdheim-Chester disease
Periadventitial tissue
Retroperitoneal fibrosis
Sclerotic bone lesions
Chiocchi, M., Luciano, A., De Stasio, V., Pugliese, L., Di Donna, C., Cerocchi, M., et al. (2023). Radiological findings in Erdheim Chester disease: A very rare multisistemic disease. RADIOLOGY CASE REPORTS, 18(5), 2047-2054 [10.1016/j.radcr.2023.02.063].
Chiocchi, M; Luciano, A; De Stasio, V; Pugliese, L; Di Donna, C; Cerocchi, M; Gigliotti, P; Carini, A; Chirico, F; Camedda, R; Di Biagio, D; Sbordone,...espandi
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2108/329756
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